Treatment of Hemophilia

November 12, 2012

Treatment of Hemophilia

Hemophilia usually cause premature death in patients, many of those being children.

Until as recently as a few decades ago many hemophilia patients suffered a premature death as a result of their condition. Unfortunately many of these deaths were in children – the result of injury or surgery.

However, in recent yearÂ’s hemophilia treatment has advanced to such an extent that most patients with the condition have a normal life expectancy.

Treatment breakthrough

Previously hemophilia treatment included blood or plasma transfusions to control any bleeding episodes and even though this did alleviate the condition it was only partial treatment. Using transfusions did not enable clotting factor levels to be sustained or even to reach effective levels.

From 1965 onwards cryoprecipitate became the first really effective hemophilia treatment. A freeze dried concentrate with the right levels of clotting factors, which could be kept at home and used as required, became available in the late 1960s and meant huge improvements in quality of life for hemophilia sufferers. Unfortunately the products used at this time were not screened and a large number of recipients became infected with HIV, Hepatitis B and Hepatitis C.

From the mid 80s stringent screening was put in place which meant the risk of viral transmission through blood products was reduced to almost zero. By the 90s synthetic factors started to become widely available.

When the coagulation factor deficiencies which are linked to hemophilia were identified they were then able to be replaced by using products from human blood.

Treatment

Factors affecting hemophilia treatment vary according to the severity of the condition and will involve clotting factor replacement therapy.

  • On demand treatment is common for patients with mild hemophilia and is administered to stop bleeding as it occurs.

  • Preventative treatment for patients with moderate or severe hemophilia includes medication to prevent episodes of bleeding and complications which may include joint or muscle damage.

Clotting factors

There are two methods of preparing the clotting factor concentrates used in hemophilia treatments -

  • Plasma derived clotting factors, from the plasma of donated human blood

  • Recombinant clotting factors, produced from using a combination of synthetic, human and animal products.

Desmopressin

  • Desmopressin is a synthetic hormonal medication prescribed to patients with hemophilia A in order to encourage the production of Factor VIII – although in the event of a serious bleed factor VIII replacement therapy may still be necessary.

Rest, Ice, Compression, Elevation (RICE)

Recommended for joint bleeds and in conjunction with clotting factor concentrates will reduce tissue damage and swelling.

Administration of clotting factor concentrates

Hemophilia treatments can generally be self-administered, or administered by a carer, by injection of the medication into a vein in the back of the hand or in the crook of the elbow, these treatments will stop bleeding effectively and rapidly.

Bleeding episodes

Bleeds are inevitable for hemophilia sufferers, even those with a mild condition; because the issue is prolonged bleeding rather than rapid bleeding the situation may not necessarily be perceived as a medical emergency. However medical treatment should be sought immediately in the following situations -

  • Injury to neck, mouth, tongue, face or eye

  • Severe blow to the head

  • Heavy or persistent bleeding

  • Severe pain or swelling

  • Presence of an open wound requiring sutures.

Should there be any cause for doubt then medical assistance should be sought as soon as possible.

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